In 1943, Waldenström 1 described a new syndrome, purpura hyperglobulinemica (PH), characterized by recurrent crops of petechiae or purpura on the lower extremities, marked elevation of the erythrocyte sedimentation rate (ESR) and increased serum globulin levels, without detectable underlying disease. Despite increased recognition of the entity,2-10 its etiology and natural history remain obscure. In 1957 we reported a typical instance of PH.10 This communication reports the patient's subsequent clinical course, with additional family studies, and, we believe, provides some insight into the nature of this syndrome.
The patient, patient 1, a 47-year-old white woman, was first seen in 1955. Her symptoms dated back to 1952, when she experienced a transitory sensation of pain in the right knee. Shortly thereafter, a petechial rash appeared on the legs. The eruption slowly faded, only to recur at varying intervals of time. Its appearance was unrelated to menstrual cycle, food,